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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
09:18

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

Published on: January 12, 2019

Prognostic impact of left ventricular noncompaction in patients with Duchenne/Becker muscular dystrophy--prospective

Koichi Kimura1, Katsu Takenaka, Aya Ebihara

  • 1Cardiovascular Medicine, The University of Tokyo, Tokyo, Japan; Pharmacoepidemiology, The University of Tokyo, Tokyo, Japan.

International Journal of Cardiology
|January 22, 2013
PubMed

Insights

Left ventricular noncompaction (LVNC) is prevalent in Duchenne/Becker muscular dystrophy (DMD/BMD) patients. This condition significantly worsens cardiac function and increases mortality risk, necessitating closer monitoring by physicians.

Area of Science:

  • Cardiology
  • Genetics
  • Neurology

Background:

  • Left ventricular noncompaction (LVNC) prevalence and prognostic significance are debated.
  • Duchenne/Becker muscular dystrophy (DMD/BMD) patients have unique cardiac considerations.

Purpose of the Study:

  • To determine the prevalence of LVNC in DMD/BMD patients.
  • To clarify the prognostic impact of LVNC in this population.

Main Methods:

  • Prospective follow-up of 186 DMD/BMD patients (aged 4-64) for all-cause death.
  • LVNC presence was assessed and blinded until study completion (median follow-up: 46 months).

Main Results:

  • LVNC was identified in 35 patients; baseline characteristics were similar to controls, except for LV function.
  • LVNC patients showed a greater decrease in left ventricular ejection fraction (LVEF) during follow-up (-8.6% vs. -4.3%).
  • LVNC independently predicted a 2.67-fold increased risk of mortality (Log-rank p<0.001).

Conclusions:

  • LVNC is a prevalent finding in DMD/BMD.
  • LVNC is associated with accelerated LV dysfunction and increased mortality in DMD/BMD.
  • Increased vigilance for LVNC is recommended for neurologists and cardiologists managing DMD/BMD patients.
Abstract

Keywords:
CardiomyopathyLeft ventricular noncompactionMuscular dystrophyNeuromuscular diseasePrognosis

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